OBJECTIVE AND IMPORTANCE: Spinal cord hamartomas are infrequently mentioned in the literature. The authors present a unique report detailing the clinical presentation of a spinal cord hamartoma, with supporting radiographic and pathological data. CLINICAL PRESENTATION: A 26-year-old man presented with progressive right upper extremity weakness. Imaging studies revealed an exophytic cervical spinal cord mass. INTERVENTION: Open biopsy was undertaken and revealed tethering of the lesion to the dura. A pathological examination revealed a spinal cord hamartoma. CONCLUSION: The patient's symptoms improved postoperatively, suggesting that tethering of the spinal cord was responsible for the symptoms. Although unusual, hamartoma should be included in the differential diagnosis of an exophytic spinal cord lesion.